Management and outcome of infants and children with right atrial isomerism

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Updated: Aug 10, 2026

Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure
07:41

Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure

Published on: February 8, 2022

M Sadiq1, O Stümper, J V De Giovanni

  • 1Heart Unit, Children's Hospital, Birmingham.

Heart (British Cardiac Society)
|March 1, 1996
PubMed

Insights

Surgery for right atrial isomerism and complex congenital heart disease has high mortality, especially in neonates. Early intervention after the first month of life significantly improves long-term survival rates.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Surgery
  • Cardiac Surgery Outcomes

Background:

  • Right atrial isomerism is a complex congenital heart defect.
  • Associated complex congenital heart disease necessitates surgical intervention.
  • Outcomes for surgical management in infants and children are critical.

Purpose of the Study:

  • To evaluate surgical outcomes in children with right atrial isomerism and complex congenital heart disease.
  • To analyze the impact of age at presentation and surgical procedures on outcomes.
  • To assess long-term survival rates after surgical interventions.

Main Methods:

  • Retrospective analysis of 20 consecutive pediatric patients.
  • Patients categorized into two groups based on age at initial surgery: early (first month) vs. late (after first month).
  • Surgical procedures, presentation, and outcomes were analyzed.

Main Results:

  • Early surgery (Group A) had a high mortality (64%) with 18% long-term survival, often due to pulmonary venous obstruction and atresia.
  • Late surgery (Group B) showed improved outcomes with 78% long-term survival and no early deaths.
  • Nine patients were candidates for the Fontan operation, with seven undergoing the procedure and five surviving.

Conclusions:

  • Surgical management for right atrial isomerism with complex congenital heart disease has high mortality and is often palliative.
  • Timely surgical intervention after the first month of life significantly improves survival.
  • The Fontan procedure offers a viable option for selected patients, improving long-term outcomes.
Abstract

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